Cystathionine beta-Synthase Deficiency

Metabolites

10-Formyltetrahydrofolate

Formula: C20H23N7O7 (473.1658888)

CAS ID: 2800-34-2

2-Oxobutanoate

Formula: C4H6O3 (102.0316926)

CAS ID: 600-18-0

5-Methyltetrahydrofolate

Formula: C20H25N7O6 (459.186623)

CAS ID: 134-35-0

5'-Methylthioadenosine

Formula: C11H15N5O3S (297.089556)

CAS ID: 2457-80-9

Adenosine

Formula: C10H13N5O4 (267.09674980000005)

CAS ID: 58-61-7

Ammonia

Formula: NH3 (17.0265478)

CAS ID: 7664-41-7

AMP

Formula: C10H14N5O7P (347.06308240000004)

CAS ID: 61-19-8

ATP

Formula: C10H16N5O13P3 (506.9957476)

CAS ID: 56-65-5

Betaine

Formula: C5H11NO2 (117.0789746)

CAS ID: 107-43-7

Choline

Formula: C5H14NO (104.10753340000001)

CAS ID: 62-49-7

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Hydrogen peroxide

Formula: H2O2 (34.0054792)

CAS ID: 7722-84-1

L-Cysteine

Formula: C3H7NO2S (121.0197482)

CAS ID: 52-90-4

L-Homoserine

Formula: C4H9NO3 (119.0582404)

CAS ID: 672-15-1

L-Methionine

Formula: C5H11NO2S (149.0510466)

CAS ID: 63-68-3

L-Serine

Formula: C3H7NO3 (105.0425912)

CAS ID: 56-45-1

N,N-Dimethylglycine

Formula: C4H9NO2 (103.0633254)

CAS ID: 1118-68-9

NAD+

Formula: C21H28N7O14P2 (664.1169428000001)

CAS ID: 53-84-9

NADH

Formula: C21H29N7O14P2 (665.1247674)

CAS ID: 58-68-4

Oxygen

Formula: O2 (31.98983)

CAS ID: 7782-44-7

Putrescine

Formula: C4H12N2 (88.1000432)

CAS ID: 110-60-1

Sarcosine

Formula: C3H7NO2 (89.0476762)

CAS ID: 107-97-1

Spermidine

Formula: C7H19N3 (145.1578894)

CAS ID: 124-20-9

D-Methionine (S)-S-oxide

Formula: C5H11NO3S (165.0459616)

CAS ID: 62697-73-8

diphosphate

Formula: O7P2 (173.911931)

CAS ID: 14000-31-8

phosphate(3-)

Formula: O4P (94.953423)

CAS ID: 14265-44-2



Enzyme

EC Number name full name note
1.1.99.1 choline dehydrogenase choline:acceptor 1-oxidoreductase
1.4.3.2 L-amino-acid oxidase L-amino-acid:oxygen oxidoreductase (deaminating)
1.4.3.25 L-arginine oxidase L-arginine:oxygen oxidoreductase (deaminating)
1.5.1.53 methylenetetrahydrofolate reductase (NADPH) 5-methyltetrahydrofolate:NADP+ oxidoreductase
1.8.4.12 peptide-methionine (R)-S-oxide reductase peptide-methionine:thioredoxin-disulfide S-oxidoreductase [methionine (R)-S-oxide-forming]
1.8.4.14 L-methionine (R)-S-oxide reductase L-methionine:thioredoxin-disulfide S-oxidoreductase [L-methionine (R)-S-oxide-forming]
2.1.1.37 DNA (cytosine-5-)-methyltransferase S-adenosyl-L-methionine:DNA (cytosine-5-)-methyltransferase
2.1.1.5 betaine-homocysteine S-methyltransferase trimethylammonioacetate:L-homocysteine S-methyltransferase
2.1.2.1 glycine hydroxymethyltransferase 5,10-methylenetetrahydrofolate:glycine hydroxymethyltransferase
2.4.2.28 S-methyl-5'-thioadenosine phosphorylase S-methyl-5'-thioadenosine:phosphate S-methyl-5-thio-alpha-D-ribosyl-transferase
2.5.1.16 spermidine synthase S-adenosyl 3-(methylthio)propylamine:putrescine 3-aminopropyltransferase
2.5.1.6 methionine adenosyltransferase ATP:L-methionine S-adenosyltransferase
4.1.1.50 adenosylmethionine decarboxylase S-adenosyl-L-methionine carboxy-lyase [(5-deoxy-5-adenosyl)(3-aminopropyl)methylsulfonium-salt-forming]
4.2.1.22 cystathionine beta-synthase L-serine hydro-lyase (adding homocysteine; L-cystathionine-forming)
4.4.1.1 cystathionine gamma-lyase L-cystathionine cysteine-lyase (deaminating; 2-oxobutanoate-forming)
4.4.1.2 homocysteine desulfhydrase L-homocysteine hydrogen-sulfide-lyase (deaminating; 2-oxobutanoate-forming)
6.1.1.10 methionine-tRNA ligase L-methionine:tRNAMet ligase (AMP-forming)


Proteins

Protein ID name full name